Causes of rib notching

1. RibNotching
Dr. Y. Madhu Madhava Reddy

2. Introduction
• Rib notching refers to deformation of the
superior or inferior surface of the rib.
• It can affect single ribs (from trauma or
solitary masses e.g. schwannoma) or can
affect multiple ribs.
• The differential differs according to whether it
is the superior or inferior surface that is
notched.

3. Differentials
• Superior rib notching:
• The superior rib notching can be caused by
1. Abnormal osteoblastic activity
– osteogenesis imperfecta
– connective tissue diseases
• rheumatoid arthritis
• systemic lupus erythematosus (SLE)

4. Superior rib notching
2. Abnormal osteoclastic activity
– hyperparathyroidism
3. Miscellaneous
– neurofibromatosis type 1
– restrictive lung disease
– Poliomyelitis
– Marfan syndrome

5. Inferior rib notching
• Aka Roesler sign
1. Enlarged collateral vessels
– coarctation of the aorta
– interrupted aortic arch
– subclavian artery obstruction
• Takayasu disease
• Blalock-Taussig shunt : involves only upper 2 rib spaces
– AVM of the chest wall
– SVC obstruction with enlarged venous collaterals
– pulmonary AVM

6. Inferior rib notching
2. Neurogenic tumours
– schwannoma (usually single)
– neurofibromatosis type 1 (rarely can be superior if
neurofibroma is very large)

7. Superior and Inferior Rib notching
• 3. Superior and Inferior Rib notching
- hyperparathyroidism

8. Superior rib notching
• Osteogenesis imperfecta:
• The hallmark feature of osteogenesis
imperfecta is that fragile bones that fracture
easily. It affects both bone quality and
quantity.
• It is due to abnormality of type 1 collagen, so
sclera, cornea, joints, and skin are abnormal.
• Due to the abnormal osteoblastic activity the
superior part of ribs is deformed.

9. Types of OI
• Type 1: Most common.
• Sclera are blue, Bony fragility is mild, Stature is
reduced, Deafness can occur in adult life.
• Osteoporosis occur with cortical thinning.
Long Bones are bowed and gracile.

10. OI

11. Types of OI
• Type 2: Gross demineralisation of long bones
with thin cortices. Numerous healing or
healed rib fractures are seen.
• Type 2A: Long bones are bowed, short and
broad. Numerous fractures are seen. Ribs are
broad with continuous beading.
• Type 2B: Long bones as in case of type 2A, but
ribs show less or no beading.

12. Types of OI
• Type 2C: the long bones are thinned, show
numerous fractures and ribs are too thin and
beaded.
• Type 3: over all bones are demineralised,
Vertebral compression is seen, kyphoscoliosis
may present.
• Sutures may be wide and wormian bones
persists. Associated with dentinogenesis
imperfecta.

13. Types of OI
• Type 4: the ribs are so soft and thin that the
down ward pull of the intercostal muscles
make their posterior portion convex
downward.

14. OI

15. OI

16. ZEBRA LINES IN BISPHOSPHONATE
THERAPHY

17. Rheumatoid Arthritis
• It is a chronic systemic inflammatory disease
which affects many organs, but predominantly
attacks the synovial tissues and joints.
• Proximal joints are affected first, with
symmetrical involvement of joints.

18. Rheumatoid Arthritis
• Diagnosis is based on a combination of clinical,
radiographic and serological criteria.
1. Morning stiffness lasting at least 1 hour before
maximal improvement
2. Soft tissue swelling of 3 or more joints observed by a
physician
3. Swelling of the proximal interphalangeal,
metacarpophalangeal, or wrist joints
4. Symmetric swelling
5. Rheumatoid nodules
6. The presence of rheumatoid factor; and
7. Radiographic erosions and/or periarticular osteopenia
in hand and/or wrist joints.

19. Rheumatoid Arthritis
• The erosions are typical being localized
defects in the superior aspect of the upper
ribs towards their posterior ends. These
erosions occur symmetrically and involve each
particular rib at a constant distance from the
costo verterbral joint.

20. RA

21. RA Clavicular erosion

22. SLE
• It is a complex autoimmune disease with
multisystem involvement.
• Diagnosis is made using the following criteria:
• Malar rash, discoid rash, photo sensitivity,
pleuritis / pericarditis etc.
• SLE affects bone formation and bone strength
there by causing the rib notching.

23. Marfan syndrome
• Marfan syndrome is a multi system hereditary
connective tissue disease with a high
penetrance and variable expression.
Pathology:-
• Results from a defect in fibrillin 1 (FBN1) gene
located in chromosome 15 which is
responsible for cross linking collagen.

24. Disease spectrum and associated
features
Skeletal:-
• General
– tall stature
– osteopaenia
– joint laxity
• recurrent dislocations

25. Marfan syndrome
• Spine / skull
– High arched palate
– Atlanto-axial subluxation
– Dural ectasia
– Increased interpedicular distance
– kyphoscoliosis
– Enlarged sacral foramina
– Meningocoele
• pre sacral meningocoele
• lateral sacral meningocoele
– Vertebral scalloping

26. Marfan syndrome
Cardiovascular:-
• Arterial dissection
• Aortic aneurysm
• Aortic valve regurgitation (AR)
• Aortic coarctation
• Aortic sinus dilatation
• Myxomatous degeneration of the mitral valve
• Pulmonary arterial dilatation

27. Marfan syndrome
Ocular:-
• Ectopia lentis lens usually displaced upwards
and out
• Myopia
• Retinal detachment
• Megalocornea

28. Hyperparathyroidism
• It is the effect of excess parathyroid hormone
in the body.
• Increased levels of parathyroid hormone (PTH)
lead to increased osteoclastic activity. The
resultant bone resortpion produces cortical
thinning (subperiosteal resorption) and
osteopaenia. This leads to the rib notching on
Chest X ray.

29. Hyperparathyroidism
• Hyperparathyroidism causes both superior
and inferior rib notching due to the increased
levels of PTH in blood initiating the
osteoclastic reaction.

30. Inferior rib notching (Roesler sign)
• Enlarged collateral vessels:-
– Coarctation of the aorta
– Interrupted aortic arch
– Subclavian artery obstruction
• Takayasu disease
• Blalock-Taussig shunt: involves only upper 2 rib spaces
– AVM of the chest wall
– SVC obstruction with enlarged venous collaterals
• Neurogenic tumours:-
– Schwannoma (usually single)
– Neurofibromatosis type 1 (rarely can be superior if
neurofibroma is very large)

31. Coarctation of the aorta
• Coarctation of the aorta refers to a narrowing
of the aortic lumen.
It can be primarily divided into two types
• Infantile (pre-ductal) form
• Adult (juxta-ductal, post-ductal or middle
aortic) form

32. Coarctation of the aorta
• Figure of 3 sign - contour abnormality of the aorta
• Inferior rib notching - Roesler sign
– Secondary to dilated intercostal collateral vessels
– Seen only in long standing cases, and therefore not
seen in infancy
– Seen in 70% of cases presenting in older children or
adults
– If unilaterally seen on the left, then this suggests an
associated aberrant right subclavian artery arising
after the coarctation.

33. Coarctation of the aorta
– If unilaterally seen on the right, then the origin of the
left subclavian artery is distal to the coarctation.
– Most often involves 4th-8th ribs
– Occasionally involves 3rd and 9th ribs
– Does not involve 1st and 2nd ribs (the associated arteries
are branches of the costocervical trunk, and thus proximal
to coarctation)

34. Coarctation of the aorta
• Coarctation of the aorta with an aberrant
right subclavian artery: Left-sided rib notching
is seen. Occurs when the aberrant right
subclavian artery arises after the coarctation.
• Coarctation of the aorta proximal to the left
subclavian artery: Right-sided rib notching
occurs.

35. Coarctation of the aorta
• Rib notching in Coarctation of aorta occurs
due to dilatation of the posterior intercostal
arteries, which act as collateral vessels.
• Since 1st and 2nd posterior intercostal arteries
arise from the costocervical trunk of sub
clavian artery rather than descending aorta,
they do no form a collateral path and hence
do no cause rib erosion.

36. COA

37. FIGURE OF 3

38. COA

39. COA

40. Interrupted aortic arch
• It is an uncommon congenital cardiovascular
anomaly where there is a separation between the
ascending and descending aorta .
• It can either be complete or connected by a remnant
fibrous band .
• An accompanying large ventricular septal defect (VSD) and
or patent ductus arteriosus (PDA) is frequently present.
Pathology
• Faulty embryological development of the aortic arch
(thought to occur during the 5th to 7th week of intra uterine
life).

42. Classification:-
• Type A : second commonest : interruption occurs distal to the
left subclavian arterial origin
• Type B : commonest (> 50 %) : interruption occurs between
left common carotid arterial and left subclavian origins
• Type C : rare : interruption occurs proximal to left common
carotid arterial origin
Each type is divided into 3 subtypes :
• sub-type 1 : normal subclavian artery
• sub-type 2 : aberrant subclavian artery
• sub-type 3 : isolated subclavian artery that arises from the
ductus arteriosus.

43. Type A : second commonest : interruption occurs
distal to the left subclavian arterial origin
Type B : commonest (> 50 %) : interruption
occurs between left common carotid arterial and
left subclavian origins
Type C : rare : interruption occurs proximal to left
common carotid arterial origin

44. Abberant Right Subclavian Art.

45. Radiographic features
Plain film - CXR
• Plain film features are often non specific
• The aortic knuckle may be absent
• May show cardiomegaly
Antenatal ultrasound
• The right ventricle may appear a lot larger than the left
The ascending aorta may also appear more vertical
than usual.
MRI / MRA
• Non visualisation of portion of interruption
• Great vessels may show a "V" configuration on coronal
imaging 2

46. IAA

47. Subclavian artery obstruction
– Takayasu disease
– Blalock-Taussig shunt : involves only upper 2 rib
spaces

48. Takayasu arteritis
• Takayasu arteritis (TA) (also known
as idiopathic medial aortopathy or pulseless
disease) is a granulomatous large
vessel vasculitis predominantly affecting
the aorta and its major branches.
• It may also affect the pulmonary arteries.

49. Takayasu arteritis
Pathology
• There is segmental and patch granulomatous
inflammation of the aorta which results in
stenosis, thrombosis and aneurysm formation.
Half of patients present with an initial
systemic illness whereas the other 50%
present with late-phase complications.

50. Takayasu arteritis
Two phases of the disease are classically
described :-
• Pre pulseless phase : characterised by
nonspecific systemic symptoms
• Pulseless phase : presents with limb ischaemia
or renovascular hypertension.

51. Takayasu arteritis
• The initial systemic illness may include symptoms of
malaise, fever, night sweats, weight loss and arthralgia.
There is often anaemia with raised inflammatory
markers.
• This phase gradually resolves with initiation of the
chronic phase which is characterised by inflammatory
and obliterative changes in the aorta and its branches.
• There are often reduced or absent peripheral pulses,
giving rise to it's alternative name of "pulseless disease

52. Takayasu arteritis
• Subtle notching of the first three ribs may be
an expression of sub clavian artery occlusion.
• Nothing of the lower ribs is usually associated
with severe narrowing or occlusion of the
upper abdominal aorta with intercostal
arteries serving as prominent collateral
channels.

53. Location and classification
• Type I - classic type involving the solely the aortic arch branches :
brachiocephalic trunk, carotid and subclavian arteries
• Type II -
– IIa - involvement of the aorta solely at its ascending portion and/or
at the aortic arch +/- branches of the aortic arch
– IIb - involvement of the descending thoracic aorta +/ - ascending or
aortic arch + branches
• Type III - involvement of the thoracic and abdominal aorta distal to the
arch and its major branches (i.e. descending thoracic aorta +
abdominal aorta +/ - renal arteries)
• Type IV - sole involvement of the abdominal aorta and/or the renal
arteries
• Type V - generalised involvement (all aortic segments)

54. Radiographic features
Ultrasound :-
• Homogeneous circumferential thickening of
affected vessels (indistinguishable from
atherosclerotic plaque)
• Vascular occlusion +/- dilation
• Flow velocity elevations beyond stenotic
segments

55. CT/MRI:-
• Wall thickening - acute active phase
• Wall enhancement - acute active phase
• Aortic valve disease - stenosis, regurgitation
• Occlusion of major aortic branches
• Aneurysmal dilatation of the aorta or its
branches.
• Pseudoaneurysm formation
• Diffuse narrowing distally (i.e. descending and
abdominal aorta ) - in late phase

56. Blalock-Taussig shunt
• Blalock-Taussig shunt: involves only upper 2 rib spaces.
• A Blalock-Taussig (BT) shunt is a palliative
procedure performed in patients with tetralogy of Fallot (prior
to the ability to repair the defect) to increase the pulmonary
blood-flow.
• The shunt sacrificed the subclavian artery (with a distal
ligation) and the proximal portion is routed downwards to an
end to side anastomosis with the ipsilateral branch of
the pulmonary artery.

57. Blalock-Taussig shunt

58. Blalock-Taussig shunt
• Rib notching is Ipsilateral to the side of shunt.
Due to division of all the branches of the first
part of the subclavian artery performed during
shunt creation.

60. Points to remember
• Coarctation of aorta proximal to left
subclavian artery : Right sided rib notching
• Coarctation of aorta with an abberant right
subclavian artery: left sided rib notching
• Sub clavian stenosis: ipsilateral to the side of
stenosis.
• Blalock taussig shunt: ipsilateral to the side of
shunt.

61. Superior vena caval obstruction
• Superior vena caval obstruction can occur
from of a wide variety of pathologies.
• Superior vena caval syndrome refers to the
clinical syndrome with symptoms that results
from obstruction of the superior vena cava.

62. Causes include: -
• Malignancy, e.g. lung cancer (the most
common cause)
• Central venous catheters
• Pacemaker wires
• Fibrosing mediastinitis
• Due to development of collateral channels to
the inferior vena cava via the intercostal veins
rib notching occurs in cases of SVC obstruction

63. • Neurogenic tumours:-
– Schwannoma (usually single)
– Neurofibromatosis type 1 (rarely can be superior if
neurofibroma is very large)
– They arise from the intercostal neurovascular
bundles. They grow large in size causing rib
notching.

64. NeuroVascular Bundle

65. Neurogenic tumor

66. NF 1

67. RIBBON RIBS

68. Bilateral rib notching

69. PROGERIA

71. Thank you